Elevated Hepatic Copper Content in Porto-Sinusoidal Vascular Disorder (PSVD): Leading Down a Wrong Track

Area of Science:

• Hepatology
• Vascular Liver Disorders
• Wilson Disease Differential Diagnosis

Background:

• Porto-sinusoidal vascular disorder (PSVD) is a rare liver condition with unknown pathophysiology.
• Elevated hepatic copper is a hallmark of Wilson disease (WD), but its role in PSVD is unexplored.

Purpose of the Study:

• To investigate hepatic copper content in patients diagnosed with PSVD.
• To correlate hepatic copper levels with clinical features and patient outcomes in PSVD.

Main Methods:

• Retrospective analysis of 92 PSVD patients with available hepatic copper content.
• Correlation of hepatic copper levels with cholestatic changes, WD diagnostics, and liver-related outcomes.

Main Results:

• 32% of PSVD patients exhibited moderately elevated hepatic copper (≥50 μg/g), and 4% had strongly elevated levels (≥250 μg/g).
• Elevated hepatic copper was associated with younger age and increased risk of liver-related outcomes (aHR: 1.60).
• A hepatic copper cut-off of ≥90 μg/g identified PSVD patients with a significantly higher risk of adverse liver outcomes.

Conclusions:

• Elevated hepatic copper is frequent in PSVD, even without cholestasis, complicating WD differential diagnosis.
• Increased hepatic copper in PSVD patients correlates with a higher risk of liver-related outcomes.
• Further research into the mechanisms of hepatic copper accumulation in PSVD may reveal new therapeutic targets.